Hepatoblastoma Flashcards
What conditions are associated with increased risk of hepatoblastoma?
- familial adenomatous polyposis
- Beckwith-Weidemann syndrome (associated with Wilms, RMS, adrenocorticocarcinoma)
- very low birth weight
- trisomy 18
What are some genetic causes of HB?
- FAP
- gardner syndrome
- BWS
- hemihyperplasia
- T18
- glycogen storage disease
What does Pretext staging describe?
The number of continuous sections which are tumour free
COG stage I
complete gross resection at diagnosis
COG stage II
complete gross resection with residual margins
COG stage III
incomplete gross resection, biopsy only, nodes or spill/rupture
COG stage IV
distant metastases
Treatment of standard risk HB
Single agent cisplatin Q 2 weeks x 6
How do you manage locally unresectable HB? (s/p neoadjuvant chemo)
hepatic transplant
Oto-protectant medications
Amifostine
STS
Cardioprotectants
Dexrazoxane
Treatment of low-risk pretext I/II s/p resection
C5V x 4 Q3wks
Treatment of high-risk (SCU, AFP<100 or pretext IV or metastatic)
SuperPLADO, cisplatin x 5, carbo/doxo x 5 Q2 weeks
AFP half-life?
5-7 days
what gene is mutated in familial adenomatous polyposis (FAP)?
APC
what is the most commonly mutated gene in hepatoblastoma
CTNNB1
what is the SIOPEL staging system?
PRETEXT - based upon number of continuous uninvolved regions of liver.
PRETEXT 1 = 3 uninvolved regions
PRETEXT 2 = 2 uninvolved
PRETEXT 3 = 1 uninvolved
PRETEXT 4 = all regions involved
*there are specific annotations for additional risk factors (ie, venous, portal involvement, LN, tumour rupture, mets etc)
What is the COG/Evan’s staging system?
Stage 1 - no mets, tumour completely resected
Stage 2 - no mets, microscopic residual tumour (ie, + margins, rupture etc)
Stage 3 - no mets, gross residual tumour
Stage 4 - mets
SIOPEL risk grouping
Standard risk - PRETEXT I, II or III
High risk:
- small cell undifferentiated
- AFP < 100
- tumour rupture
COG risk grouping
very low risk = PRETEXT I or II with fetal histology with resection at diagnosis
low/standard risk = PRETEXT I or II, any histology, with resection at diagnosis
intermediate risk = PRETEXT II, III or IV unresectable at diagnosis (+V, +P or +Extrahepatic spread), SCU histology (AFP > 100)
high risk = any PRETEXT with metastasis, AFP < 100
how do you stage a liver tumour?
need high-quality cross sectional imaging (MRI + contrast preferred) to determine PRETEXT group and annotation factors
What is the cause of Beckwith-Wiedemann syndrome?
11p15 paternal uniparental disomy
how do you screen for hepatoblastoma?
ultrasound and AFP q3 months from diagnosis to 4th birthday
what intracellular pathway is aberrantly activated in hepatoblastoma and why
WNT pathway (usually related to CTNNB1 activating mutations/deletions)
